Cancer, Chromaffin Cell (Pheochromocytoma)


Article Author:
Ateeq Mubarik


Article Editor:
Narothama Aeddula


Editors In Chief:
Juan Batlle
Jitendra Sisodia
Jeffrey Miller


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
3/14/2019 4:45:26 PM

Introduction

The term pheochromocytoma first described by Mr. Pick in 1912. Pheochromocytoma is a tumor that arises from chromaffin cells present in the adrenal medulla or paraganglion cells. It named in this way as these tissues have a special reaction on the application of a chromium salt. Dr. Charles Mayo presented the first case of pheochromocytoma in 1937 with a clinical diagnosis and therapeutic surgical resection.

Etiology

In 90% of the cases, pheochromocytoma is sporadic, but in 10% of patients, it is familial and associated with syndromes like Von Hippel–Lindau syndrome, type 1 neurofibromatosis, and multiple endocrine neoplasia syndromes type IIA and type IIB with an autosomal dominant mode of transmission.[1]

Epidemiology

Pheochromocytoma is a rare neuroendocrine disorder. Its incidence is about 0.1% to 0.9% but only 0.1% to 0.3% in the hypertensive population.[2][3] The annual incidence is approximately 0.8 per 100,000 person-years.[4] Its incidence is equal in both genders. It's usually diagnosed between the age of 30 to 50, but pheochromocytoma due to genetic predisposition can present earlier.

Pathophysiology

Pheochromocytoma is a catecholamine-secreting neuroendocrine tumor. It is 1 of the 3 types based on secreting catecholamine.

  • Norepinephrine secreting tumor
  • Epinephrine secreting tumor
  • Norepinephrine and epinephrine secreting tumor

Tumors secrete only norepinephrine usually presents with sustained hypertension. Norepinephrine and epinephrine secreting tumor present with paroxysmal hypertension. Only epinephrine can cause hypotension instead of hypertension.[5]

Due to the release of catecholamine, there is an increase in heart rate, systemic vascular resistance, inotropic effect, and a decrease in venous compliance. In pheochromocytoma, there is orthostatic hypotension as it is volume depleted type of hypertension.

Histopathology

Macroscopically, pheochromocytoma is encapsulated and appears brown to yellow and soft. It comprises of cystic degeneration, patches of necrosis, or hemorrhage.

Histopathological examination showed that pheochromocytoma is composed of large basophilic cells assembled as a nest; the cytoplasm comprises multiple granules with many large, pleomorphic, and bizarre nuclei. The nuclei have prominent nucleoli and coarse chromatin. Occasional intracytoplasmic hyaline globules positive for periodic acid–Schiff stain are present. There is a thin rim of a subcapsular lipid-rich adrenal tissue. There is no clear demarcation between the tumor cells and normal adrenal tissue.

Hyperchromasia, nuclear pleomorphism, or capsular or vascular invasion may be present, but these are not a predictor of malignancy. Metastases to a different site are the only indicator of malignancy.

History and Physical

Pheochromocytoma can be asymptomatic and diagnosed by further workup of an adrenal incidentaloma. It can present with vague symptoms like a headache (50%), palpitations (60%), and diaphoresis (50%) that can lead to a paroxysmal hypertensive crisis due to increased catecholamine production. Other associated symptoms include pallor (40%), nausea (40%), tremor, trembling, fatigue (40%), anxiety, fever, pain, and flushing (20%).[6] Characteristically, these symptoms are paroxysmal and may be precipitated by abdominal exertion, such as heavy lifting or performing the Valsalva maneuver. Almost 90% of the population with pheochromocytoma has hypertension.  Pheochromocytoma follows the rule of 10 which means only 10% are malignant, 10% are extra-adrenal, and 10% are bilateral, extra-abdominal, familial, pediatric, and without hypertension. Recently, the diagnosis of asymptomatic people with pheochromocytoma has increased due to modern imaging techniques and intensive genetic testing.

Pheochromocytoma can also cause hyperglycemia (40%), lactic acidosis, and weight loss.

Evaluation

If there is clinical suspicion of a pheochromocytoma, then initial assessment starts with biochemical laboratory testing.[7] Plasma free metanephrine or 24-hour urinary fractioned metanephrine testing is recommended; both can be done as an initial screen. The sensitivity is almost the same between these two biochemical tests, but there is a difference in the specificity. The plasma test (89%) is more specific than a urine test (69%).[8] Urinary vanillylmandelic acid is also used, but it is not as sensitive (64%).

The discussion above indicates that the best initial screening test is the plasma-free metanephrine biochemical test.[8] Amphetamines, ephedrine, tricyclic antidepressants, and cocaine can affect plasma and urine metanephrine levels; therefore, abstinence of these substances has been suggested prior to these biochemical tests.[9] It is recommended that the clinician collect plasma samples on ice, with the patient in a supine position, and in a fasting state to decrease the incidence of false-positive results.[10]

Once metanephrine levels are 4-fold high from the normal reference value, then the next goal is to localize the hormone-secreting tumor. The problem arises when the metanephrine levels are minimally elevated or equivocal.[9] In that circumstance, the clonidine suppression test can help with the diagnosis.[11]

Computed tomography (CT) scan or magnetic resonance imaging (MRI) can be used for localizing the tumor. In certain conditions like pregnancy, contrast allergy, and the pediatric population, MRI should be done instead of a CT scan. Thes imaging modalities are sensitive but not specific. A 123I-labeled metaiodobenzylguanidine (MIBG) scintigraphy is very specific for catecholamine-secreting tumors. It not only helpful in differentiating between adrenal and paraganglionic tumor but also helps in revealing multiple lesions and metastases.[12] 

Positron emission tomography (PET) scan is superior to MIBG especially in the evaluation of metastatic disease.[13]. So far, genetic testing depends on a case to case basis and shared decision making. Indications for genetic testing include a family history of catecholamine-producing tumor, age less than 50 years old, multiple tumors at different sites, metastases, or bilateral tumors.[14]

Treatment / Management

The gold standard treatment of pheochromocytoma is surgical resection. An interprofessional team is required for management.[15] It is mandatory to block catecholamine effect before surgery to prevent hypertensive crises and arrhythmias even in the patients who are normotensive or asymptomatic. There is no particular preference for one drug over another to prevent hypertensive crises. Any alpha-adrenoceptor antagonist, a calcium-channel blocker, or angiotensin-receptor blocker can be used.[16] Calcium channel blockers or B-adrenergic blockers are indicated for the prophylactic treatment of arrhythmias.[17]

The surgical technique depends on the size and site of the tumor.[18] Usually, tumors less than 10 cm are removed by laparoscopic surgery.[19] If there is bilateral disease, then selective adrenal medulla surgery is suggested to prevent the use of lifelong glucocorticoids, but this technique also increases the risk of recurrence.[20][21]

Differential Diagnosis

There are a variety of diseases similar to pheochromocytoma in clinical presentation.

Endocrine

Hyperthyroidism, carcinoid tumor, hypoglycemia, medullary thyroid carcinoma, mastocytosis, menopausal syndrome

Cardiovascular

Heart failure, arrhythmias, ischemic heart disease

Neurological

Migraine, stroke, epilepsy, meningioma, postural orthostatic tachycardia syndrome (POTS)

Miscellaneous

Porphyria, panic disorder or anxiety, Factitious disorders (from the use of sympathomimetic drugs such as ephedrine), drug treatment (monoamine oxidase inhibitors, sympathomimetic drugs, withdrawal of clonidine), use of illegal drugs (cocaine)

Radiation Oncology

MIBG negative metastatic tumors have a very poor prognosis. 123I-MIBG positive tumors can be treated with MIBG therapy in the form of 131I-MIBG, which emits beta radiation. There is no reliable data about its efficacy due to lack of comparable study designs.

Medical Oncology

The metastatic pheochromocytoma has no curative treatments unless the site of disease is surgically resectable. Ten percent of all catecholamine-secreting tumors are malignant and are histologically and biochemically the same as benign tumors.

Palliative chemotherapy includes cyclophosphamide, vincristine, dacarbazine, and temozolomide along with other symptomatic therapy can increase the median survival from 2 to 26 months. [22]Further studies regarding the use of sunitinib (tyrosine kinase inhibitor) and mTOR inhibitors are underway.

Prognosis

Usually, surgery is curative for benign or non-metastatic tumors. Metastatic tumors have a 50% survival over 5 years.[15]

Complications

Pheochromocytoma can induce the Takotsubo syndrome.[23]

Pheochromocytoma crisis (severe acute presentation of a catecholamine-induced hemodynamic instability causing end-organ damage). There are two types:

  1. Type A: End organ damage but in the absence of shock
  2. Type B: End organ damage with shock

Pheochromocytoma crisis though rare can be a potentially life-threatening emergency. Crisis can occur spontaneously or precipitated by the administration of pharmacological agents, such as glucagon, ergotamine, and caffeine, tumor manipulation, micturition (urinary bladder pheochromocytoma), or foods that aggravate release of catecholamines by the tumor.[15][24]

Postoperative and Rehabilitation Care

There are no American guidelines regarding the follow up of the patient postoperatively. It is suggested that repeating biochemical tests be done after 14 days of tumor surgery. However, even if the result is normal, it does not mean that the tumor is completely resected. If plasma-free metanephrine or urinary fractioned metanephrine levels are elevated for about 3 months after surgical removal, then this is an indication for repeat imaging.[21]

European guidelines recommend annual, postoperative follow-up for 10 years with biochemical testing for any recurrence or metastasis. Patients who have familial pheochromocytoma should be followed annually for a lifetime. These recommendations are for patients who have abnormal biochemical tests preoperatively. Chromogranin A measurement should be used in the population having normal, preoperative, biochemical tests.[21]

Consultations

  • Nephrology
  • Endocrinology
  • General or endocrine surgery

Enhancing Healthcare Team Outcomes

Pheochromocytoma is a rare neuroendocrine tumor with serious and lethal clinical and cardiovascular complications due to the effects of secreted catecholamines. There needs to be close coordination between the primary care provider, nephrologist and endocrinologist. In patients with diagnosed pheochromocytoma, all patients should undergo resection of the pheochromocytoma following medical preparation and coordination with the general surgeon is highly recommended.


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Cancer, Chromaffin Cell (Pheochromocytoma) - Questions

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A patient with pheochromocytoma has a blood pressure of 268/180 mmHg. Which medication should be administered?



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What is the best method for diagnosing pheochromocytoma in the pregnant patient who has unexplained hypertension or fever within 48 hours after delivery?



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Which of the following causes elevated levels of vanillyl mandelic acid?



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Which of the following medications is typically avoided in patients with pheochromocytoma due to the risk of hypertensive crisis?



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Which is least likely to be elevated in patients with a pheochromocytoma?



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Which of the following will be least affected in a patient with pheochromocytoma?



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In the early postoperative period after excision of a pheochromocytoma, which complication is least likely?



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A 9-year-old child was diagnosed with severe hypertension. On examination he has palpitations and irregular pulses. What is the most likely cause of his symptoms?



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How long prior to elective surgery should a patient with pheochromocytoma be treated with phenoxybenzamine?



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A patient with pheochromocytoma induced hypertension will have which of the following?



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Which clinical feature is not associated with pheochromocytoma?



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Tachycardia in a patient with pheochromocytoma is treated with which of the following?



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Which of the following medications should be used for pheochromocytoma associated hypertension after phenoxybenzamine is started?



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A 25-year-old male presents to the emergency department with occasional severe headaches, nausea, and vomiting. Blood pressure sitting is 230/130 mmHg with a heart rate of 90 but with standing the blood pressure drops to 200/100 mmHg and the heart rate increases to 140. A cardiovascular exam shows tachycardia without extra heart sounds. The patient's lungs are clear. The optic discs are blurred, but there are no exudates or hemorrhages. Select appropriate treatment.



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What is the best test to diagnose a pheochromocytoma?



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Select the historical fact that is not a component of the "classic" triad for pheochromocytoma.



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Select the true statement about treatment of hypertensive crisis with encephalopathy in a patient with a pheochromocytoma.



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A patient has been taking paroxetine 20 mg daily for panic attacks for the past year. She was started on lisinopril for hypertension, but it had to be discontinued due to hypotension. She is on ibuprofen for headaches that have been present for a year. She says the panic attacks are associated with headaches and are getting worse. The patient is flushed and diaphoretic with vital signs of blood pressure 180/105 mmHg lying and 113/80 mmHg standing, pulse is 110 beats/min, respiratory rate is 20, and she is afebrile. Her examination is otherwise normal. What is the appropriate diagnostic test?



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A patient reports headaches and palpitations. There is mild hypertension, increased levels of plasma norepinephrine and epinephrine, and urinary catecholamines. MRI shows an adrenal mass. What kind of cell type is most likely to be found in the tumor?



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What is the biochemical marker vanillic mandelic acid helpful to diagnose?



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A patient presents with sudden onset of diaphoresis, palpitations, and an extremely elevated blood pressure. The symptoms subside within an hour. What test should be ordered?



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What would a urinary metanephrine test be used to diagnose?



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Which of the following is the most likely outcome of intraoperative ligation of the venous drainage of the tumor during resection of a pheochromocytoma?



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What diagnosis would be considered in a patient experiencing hypertension, palpitations, and headache during their first trimester of pregnancy?



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A 40 year old male presents with a 6 month history of recurrent episodes of palpitations, headaches, and diaphoresis. Exam is normal but during an episode the heart rate and blood pressure are markedly elevated. CT scan shows a small left adrenal tumor. Select the chemical that would be elevated in the urine.



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A 10-year-old female is brought in after a fainting spell. She has had episode of headaches, palpitations, abdominal pain, and dizziness. Her mother reports the time she fainted she was pale and diaphoretic. The patient's blood pressure is 130/95 mmHg, but the rest of the exam is normal. What is the most likely diagnosis?



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Which is the first step in the diagnosis of pheochromocytomas?



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Which is the most common symptom of pheochromocytoma?



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What is the screening test for pheochromocytoma?



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From which location does pheochromocytoma originate?



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What is the next step in the work up of pheochromocytoma with positive biochemical evidence?



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Which of the following is not included in the "Rule of 10s" for pheochromocytomas?



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On non-enhanced CT, you see an adrenal mass in a hypertensive patient. Should you rule out pheochromocytoma with lab studies prior to performing a contrast enhanced scan so as to avoid the risk of inducing a hypertensive crisis?



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What is the risk of hypertensive crisis in patients with unsuspected pheochromocytomas?



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Which of the following is not correct about MRI of pheochromocytomas?



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Which radionuclide study is most helpful in evaluating pheochromocytoma?



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A patient is being prepared for administration of I-131 MIBG for a treatment of a pheochromocytoma. Which of the following should the patient receive first?



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Column chromatography for free catecholamines which shows increased catecholamine excretion is not diagnostic of pheochromocytoma because:



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A 44-year old male presents with sudden onset of high blood pressure and extreme fatigue. Two weeks ago he was found to have a blood pressure of 220/120 mmHg. He has been healthy all his life, but suddenly he developed high blood pressure. His urine test comes back positive for high levels of metanephrines. CT shows that both adrenals and kidneys appear to be normal. In which other areas should the radiologist look to determine the cause of this pathology?



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A 45-year-old man is transported to the emergency department complaining of severe anxiety. He states, "I feel like I'm gonna die." He is profusely sweating, complaining of a headache, tachycardic, pale in appearance, dyspneic, confuse, and his blood pressure is 220/110 mmHg. Based on these clinical presentations, what is the most likely diagnosis?



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A 44-year-old man comes to the provider with multiple episodes of intense sweating, headaches, and palpitations lasting for several minutes over the past month. He reports no medical history and takes no medications. Physical examination shows no abnormalities. The patient noted that during the most recent episode he measured his blood pressure at 190/140 mmHg. His blood pressure at the office today is 128/80 mmHg. Which of the following substances is most likely elevated in the patient's urine?



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A 12-year-old boy is admitted in the emergency department; medical examination shows 180/77 blood pressure, 135 beats per minute, and profuse sweating. His mother reports that he has had headaches over the past two years; the laboratory tests show abnormal levels of catecholamines. Which is the most likely diagnosis?



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Cancer, Chromaffin Cell (Pheochromocytoma) - References

References

Blake MA,Kalra MK,Maher MM,Sahani DV,Sweeney AT,Mueller PR,Hahn PF,Boland GW, Pheochromocytoma: an imaging chameleon. Radiographics : a review publication of the Radiological Society of North America, Inc. 2004 Oct     [PubMed]
Lee TH,Slywotzky CM,Lavelle MT,Garcia RA, Cystic pheochromocytoma. Radiographics : a review publication of the Radiological Society of North America, Inc. 2002 Jul-Aug     [PubMed]
Arora S,Vargo S,Lupetin AR, Computed tomography appearance of spontaneous adrenal hemorrhage in a pheochromocytoma. Clinical imaging. 2009 Jul-Aug     [PubMed]
Bryant J,Farmer J,Kessler LJ,Townsend RR,Nathanson KL, Pheochromocytoma: the expanding genetic differential diagnosis. Journal of the National Cancer Institute. 2003 Aug 20     [PubMed]
Page LB,Raker JW,Berberich FR, Pheochromocytoma with predominant epinephrine secretion. The American journal of medicine. 1969 Oct     [PubMed]
Lenders JW,Pacak K,Walther MM,Linehan WM,Mannelli M,Friberg P,Keiser HR,Goldstein DS,Eisenhofer G, Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002 Mar 20     [PubMed]
Lenders JW,Duh QY,Eisenhofer G,Gimenez-Roqueplo AP,Grebe SK,Murad MH,Naruse M,Pacak K,Young WF Jr, Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. The Journal of clinical endocrinology and metabolism. 2014 Jun     [PubMed]
Eisenhofer G,Goldstein DS,Walther MM,Friberg P,Lenders JW,Keiser HR,Pacak K, Biochemical diagnosis of pheochromocytoma: how to distinguish true- from false-positive test results. The Journal of clinical endocrinology and metabolism. 2003 Jun     [PubMed]
Madias JE, Recurrence, lingering recovery course, mild variants, and     [PubMed]
Pacak K,Eisenhofer G,Ahlman H,Bornstein SR,Gimenez-Roqueplo AP,Grossman AB,Kimura N,Mannelli M,McNicol AM,Tischler AS, Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nature clinical practice. Endocrinology     [PubMed]
Bravo EL,Tarazi RC,Fouad FM,Vidt DG,Gifford RW Jr, Clonidine-suppression test: a useful aid in the diagnosis of pheochromocytoma. The New England journal of medicine. 1981 Sep 10     [PubMed]
Furuta N,Kiyota H,Yoshigoe F,Hasegawa N,Ohishi Y, Diagnosis of pheochromocytoma using [123I]-compared with [131I]-metaiodobenzylguanidine scintigraphy. International journal of urology : official journal of the Japanese Urological Association. 1999 Mar     [PubMed]
Mann GN,Link JM,Pham P,Pickett CA,Byrd DR,Kinahan PE,Krohn KA,Mankoff DA, [11C]metahydroxyephedrine and [18F]fluorodeoxyglucose positron emission tomography improve clinical decision making in suspected pheochromocytoma. Annals of surgical oncology. 2006 Feb     [PubMed]
Amar L,Bertherat J,Baudin E,Ajzenberg C,Bressac-de Paillerets B,Chabre O,Chamontin B,Delemer B,Giraud S,Murat A,Niccoli-Sire P,Richard S,Rohmer V,Sadoul JL,Strompf L,Schlumberger M,Bertagna X,Plouin PF,Jeunemaitre X,Gimenez-Roqueplo AP, Genetic testing in pheochromocytoma or functional paraganglioma. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2005 Dec 1     [PubMed]
Lenders JW,Eisenhofer G,Mannelli M,Pacak K, Phaeochromocytoma. Lancet (London, England). 2005 Aug 20-26     [PubMed]
Proye C,Thevenin D,Cecat P,Petillot P,Carnaille B,Verin P,Sautier M,Racadot N, Exclusive use of calcium channel blockers in preoperative and intraoperative control of pheochromocytomas: hemodynamics and free catecholamine assays in ten consecutive patients. Surgery. 1989 Dec     [PubMed]
Bravo EL,Tagle R, Pheochromocytoma: state-of-the-art and future prospects. Endocrine reviews. 2003 Aug     [PubMed]
Plouin PF,Chatellier G,Fofol I,Corvol P, Tumor recurrence and hypertension persistence after successful pheochromocytoma operation. Hypertension (Dallas, Tex. : 1979). 1997 May     [PubMed]
Janetschek G,Finkenstedt G,Gasser R,Waibel UG,Peschel R,Bartsch G,Neumann HP, Laparoscopic surgery for pheochromocytoma: adrenalectomy, partial resection, excision of paragangliomas. The Journal of urology. 1998 Aug     [PubMed]
Brunt LM,Lairmore TC,Doherty GM,Quasebarth MA,DeBenedetti M,Moley JF, Adrenalectomy for familial pheochromocytoma in the laparoscopic era. Annals of surgery. 2002 May     [PubMed]
Plouin PF,Amar L,Dekkers OM,Fassnacht M,Gimenez-Roqueplo AP,Lenders JW,Lussey-Lepoutre C,Steichen O, European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. European journal of endocrinology. 2016 May     [PubMed]
Deutschbein T,Fassnacht M,Weismann D,Reincke M,Mann K,Petersenn S, Treatment of malignant phaeochromocytoma with a combination of cyclophosphamide, vincristine and dacarbazine: own experience and overview of the contemporary literature. Clinical endocrinology. 2015 Jan     [PubMed]
Beard CM,Sheps SG,Kurland LT,Carney JA,Lie JT, Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clinic proceedings. 1983 Dec     [PubMed]
Hosseinnezhad A,Black RM,Aeddula NR,Adhikari D,Trivedi N, Glucagon-induced pheochromocytoma crisis. Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists. 2011 May-Jun     [PubMed]

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